A patient with ectopic cortisol production derived from malignant testicular masses.
BACKGROUND
A 65-year-old man presented to an oncology clinic with bilateral testicular masses, lower extremity edema, and cushingoid appearance.


METHODS
Measurements of serum cortisol and adrenocorticotropic hormone levels, testicular ultrasound and abdominal CT scans, and review of histopathology to identify the cellular origin of the ectopic cortisol production.


METHODS
Cushing syndrome was diagnosed on the basis of a markedly elevated 24-hour urine free cortisol level and classic cushingoid features. The etiology of Cushing syndrome was determined to be an adrenocortical carcinoma arising from testicular adrenal rest cells. Nevertheless, the possibility of a malignant Leydig cell tumor with ectopic cortisol production could not be excluded.


RESULTS
Mitotane and metyrapone were used to decrease cortisol production. Excess mineralocorticoid activity was blocked with spironolactone; sodium retention was also managed with sodium restriction and diuretics. Despite initial success with this regimen, the patient died as a result of tumor progression and complications of poorly controlled hypercortisolism.
Jain
Shilpa H
SH
Division of Endocrinology, Diabetes, and Hypertension, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA. shjain@partners.org
Sadow
Peter M
PM
Nosé
Vania
V
Dluhy
Robert G
RG
D000314:Adrenal Rest Tumor / Q000150:complications* / Q000175:diagnosis; D018268:Adrenocortical Carcinoma / Q000097:blood / Q000175:diagnosis / Q000473:pathology; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000368:Aged; D003480:Cushing Syndrome / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D006854:Hydrocortisone / Q000097:blood*; D008297:Male; D008797:Metyrapone / Q000627:therapeutic use; D008939:Mitotane / Q000627:therapeutic use; D013736:Testicular Neoplasms / Q000150:complications* / Q000175:diagnosis
D008939:Mitotane; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone; D008797:Metyrapone